Transjugular intrahepatic portosystemic stent shunt: 11 years' experience at a regional referral centre

OBJECTIVES: Transjugular intrahepatic portosystemic stent shunt (TIPSS) is now widely used in the treatment of uncontrolled and recurrent variceal haemorrhage. This study reports the outcome and long-term follow-up of 125 patients who were referred to a single centre for TIPSS. METHODS: One hundred and twenty-five patients were referred to undergo TIPSS. All but 10 had variceal haemorrhage. The 10 patients referred with refractory ascites were excluded from the analysis. Our follow-up protocol was to assess shunt patency only if bleeding recurred or there was a clinical indication. The mean age was 51.5 years (range 18-87 years) and 64 patients (56%) were male. The commonest aetiology for chronic liver disease was alcohol (80%). At referral, 19 patients (16%) were Child-Pugh class A, 26 patients (23%) were Child-Pugh class B and 70 patients (61%) were Child-Pugh class C. The mean follow-up period was 20.4 months (range 0-95 months). RESULTS: TIPSS was successfully placed in 108 of 115 patients (94%). The thirty-day mortality was 30%. One-year and 2-year overall cumulative survival was 52% (survival ratio, 0.525; 95% confidence interval, 0.432-0.619) and 43% (survival ratio, 0.436; 95% confidence interval, 0.340-0.532), respectively. CONCLUSION: TIPSS is effective in the treatment of uncontrolled or recurrent variceal bleeding. In comparison with previously published studies, our study suggests no value in regular or routine shunt surveillance to reduce rebleeding episodes or mortality, but this needs to be further assessed in prospective randomized studies.     

Administration of N-acetylcysteine after focal cerebral ischemia protects brain and reduces inflammation in a rat model of experimental stroke

Free radicals and inflammatory mediators are involved in transient focal cerebral ischemia (FCI). Preadministration of N-acetylcysteine (NAC) has been found to attenuate the cerebral ischemia-reperfusion injury in a rat model of experimental stroke. This study was undertaken to investigate the neuroprotective potential of NAC administered after ischemic events in experimental stroke. FCI was induced for 30 min by occluding the middle cerebral artery (MCA). NAC (150 mg/kg) was administered intraperitoneally at the time of reperfusion followed by another dose 6 hr later. Animals were sacrificed after 24 hr of reperfusion. The cerebral infarct consistently involved the cortex and striatum. Infarction was assessed by staining the brain sections with 2,3,5-triphenyltetrazolium chloride. Animals treated with NAC showed a significant reduction in infarct area and infarct volume and an improvement in neurologic scores and glutathione level. Reduction in infarction was significant even when a single dose of NAC was administered at 6 hr of reperfusion. Immunohistochemical and quantitative real-time PCR studies demonstrated a reduction in the expression of proinflammatory cytokines such as tumor necrosis factor alpha (TNFalpha) and interleukin 1beta (IL-1beta) and inducible nitric oxide synthase (iNOS) in NAC compared to that in vehicle-treated animals. The expression of activated macrophage/microglia (ED1) and apoptotic cell death in ischemic brain was also reduced by NAC treatment. These results indicate that in a rat model of experimental stroke, administration of NAC even after ischemia onset protected the brain from free radical injury, apoptosis, and inflammation, with a wide treatment window.     

Three-in-one weight,height and body mass index charts for children and adults

The aim of the study was to develop four appropriate three-in-one weight, height and built in body mass index (BMI) charts, for under-fives, 0-5-year-olds, > 5-10-year-olds, > 10-18-year-olds, and adults and to delineate the normal range, underweight, overweight and obesity on the above charts. Four different charts were designed for the various age groups as indicated above. Height was made available on the x-axis, weight on the y-axis, and corresponding BMI values on the right margin. Shading of the normal range to denote the health path and marking of the cut-off curves to denote normal status, overweight, and obesity were done selecting appropriate round figures to suit both sexes in accordance with the International Obesity Task Force (IOTF) recommendations for the various age groups. Field trials were done on appropriate subjects belonging to various age groups. 500 in each group with equal male to female ratio. The field trials showed that all the studied subjects belonging to both sexes came within the purview of the chart and those with normal nutritional status, underweight, overweight, and obesity could easily be identified looking at the chart without doing any further calculation. Early intervention also could be advised as the chart could demonstrate how much weight should be gained or reduced to come within the health path. In conclusion, the charts are applicable to both sexes and are user friendly. These are appropriate for general screening of nutritional status and to determine underweight, overweight, and obesity from birth to adulthood. They give a visual display of the ideal health path with respect to weight, height, and BMI and the adjustment in weight required to reach the normal range.     

Abdominal compartment syndrome related to noninvasive ventilation

OBJECTIVE: To study the effects of noninvasive positive pressure ventilation (NIPPV) on intra-abdominal pressure. DESIGN AND SETTING: Single case report from a tertiary teaching hospital. PATIENTS AND METHODS: A 65-year-old man who experienced a sudden respiratory and cardiovascular collapse during NIPPV. This was caused by gastric overdistension due to aerophagia followed by raised intra-abdominal pressure leading to intra-abdominal hypertension and abdominal compartment syndrome. RESULTS: The respiratory and cardiovascular problems resolved immediately after the introduction of a nasogastric tube. This resulted in normalization of IAP. CONCLUSIONS: This is the first case reported of an abdominal compartment syndrome related to NIPPV. Clinicians should be aware of this possible complication while using NIPPV.     

Modified glasgow coma scale to predict mortality in febrile unconscious children

A prospective hospital based study was conducted in the Department of Pediatrics of the Kasturba Hospital, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha to predict the mortality in children admitted with fever and unconsciousness using the Modified Glasgow Coma Scale (MGCS) score. Forty eight children were admitted with fever and unconsciousness; cases of febrile convulsions, epilepsy and cerebral palsy were excluded. MGCS scores were assessed on admission and repeated at 12 hours, 24 hours, 48 hours and 72 hours after admission in each case. Diagnosis in each case was confirmed by history, examinations and investigations. All the cases were regularly followed up till death/discharge. The overall mortality was 29.1% (14/ 48) out of which 85% (12/14) died within the first 24 hours. Mortality was highest in the toddler age group and in patients with pyogenic meningitis. There was a significant association between death and MGCS scores on admission with a post test probability for discharge being only 10% with a score of less than 5 and 99% with a score of more than 10 respectively. MGCS scores on admission can be used to predict mortality in patients hospitalized with fever and unconsciousness. The scale is simple, easy, can be applied at bed side and does not need any investigations. Its application in developing countries with limited investigative and intensive care facilities can help the treating physician decide regarding referral and counseling the parents regarding the probable clinical outcome.     

Next-Generation Sequencing Based Approach to Identify Underlying Genetic Defects of Glanzmann Thrombasthenia

Glanzmann thrombasthenia (GT) is an autosomal recessive platelet function disorder characterized by mucocutaneous bleeding as the most common clinical phenotype. Patients with GT have normal platelet counts, platelet morphology but reduced platelet aggregation in response to various agonists. Homozygosity or compound heterozygosity for variants in the ITGA2B/ITGB3 genes is the genetic basis for GT. Establishing a molecular diagnosis is definitive and is important for predictive testing. Using multi-gene panels is an accurate, faster, and cost-effective mode as compared to Sanger sequencing in large genes. We used a targeted resequencing based approach to identify pathogenic variants in eight cases in seven families. These variants were validated using Sanger sequencing in patients as well as family members and were predicted probably pathogenic using in-silico prediction tools. The variants include three missense (3/7 = 43%) (ITGA2B:c.1028 T > C, ITGA2B:c.1186G > A, ITGB3:c.1388G > C), two deletions (ITGA2B:c.559delG, ITGA2B:c.3092delT), one duplication (ITGA2B:c.1424_1427dupAGGT) and nonsense variant (ITGA2B:c.2578C > T, p.Gln860Ter). Except for one case which was compound heterozygous, the rest of the cases were homozygous. We found two novel variants that are reported for the first time in GT. The targeted resequencing based approach revealed varied genetic variants in North Indian patients, including two novels ones. The high yield of our panel indicates its suitability for usage in larger cohorts for the genetic diagnosis of GT patients. This approach is cost-effective and less cumbersome as compared to Sanger sequencing for these large size genes with multiple exons. The information so obtained is helpful in prenatal testing, carrier analysis, and genetic counseling.